Abstract
RATIONALE: Primary cardiac angiosarcoma is an exceptionally rare and aggressive disease characterized by high risk of metastasis and poor prognosis. Accurate diagnosis of angiosarcoma relies on pathology. For inoperable advanced patients, biopsy is essential to confirm diagnosis and treatment strategy, but obtaining tissue samples from primary cardiac site is fraught with life-threatening hemorrhage and other procedural complications. To date, the early identification and optimal management of cardiac angiosarcoma remain formidable clinical challenges. This case report aims to evaluate the effectiveness of computed tomography-guided lung metastasis biopsy as a safer and technically feasible diagnostic alternative and to propose a multidisciplinary framework, emphasizing how coordinated care optimizes diagnostic accuracy, risk stratification, and therapeutic decision-making for this complex disease. PATIENT CONCERNS: A 34-year-old male patient was admitted to our hospital because of a cystic mass in the right atrium and multiple pulmonary nodules discovered at another hospital, alongside symptoms of increasing chest tightness. DIAGNOSES: Immunohistochemistry and gene testing confirmed the presence of angiosarcoma with lung metastases (stage IV, with NRAS mutation). INTERVENTIONS: The patient received intravenous albumin-paclitaxel (100 mg/m2). OUTCOMES: The patient was discharged after 1 cycle of albumin-paclitaxel monotherapy. One- and six-month follow-up showed that after several cycles of chemotherapy, chest tightness symptoms were improved, lung metastases significantly shrank, and heart lesions stabilized, demonstrating a good therapeutic response to albumin-paclitaxel. LESSONS: The limitation of this case report is the inevitable bias of a single sample. Further studies with larger samples are needed to investigate the efficacy of chemotherapy for patients with metastatic primary cardiac angiosarcoma.